Abstract
While a familial variety of hypernephroma is likely, the majority of cases of renal carcinoma apparently are not familial and must therefore result from differing etiologies. The occasional reports of carcinoma of the kidney in siblings or in more than one generation of the family suggest the possibility of hereditary etiologic factors in certain circumstances; however, these infrequent occurrences are insufficient to delineate the precise genetic mechanism(s). On the other hand, the occurrence of renal carcinoma in a known hereditary disorder, i.e., von Hippel Lindau's disease, is intriguing, since this autosomal dominantly inherited disorder harbors a propensity for the production of cerebellar hemangioblastoma and apparently also predisposes through unknown mechanism to the production of renal carcinoma. Renal carcinoma has been described in association with polycystic kidneys, though the frequency of this association is not accurately known. However, review of the literature suggests that it may be more common than usually credited. In von Hippel Lindau's disease cysts may occur in the liver or pancreas and kidney. In the latter organ, angiomas, adenomas, and carcinomas have been described. In tuberous sclerosis tumor like masses, or 'hamartomas', are found in several major organs, including the brain, heart, and the kidneys. The renal tumors are usually multiple, small in size, and may consist of adenomas, leiomyomas, angiomas, lipomas and fibromas, or complex mixtures comprising elements of these specific growths. Sarcomatous changes may occur on rare occasions in these renal lesions. (Vlachos - Athens)